Aetiologies and clinical patterns of hypopituitarism in Sudanese children.

There is paucity of reported information regarding aetiology and clinical profile of hypopituitarism from resource-limited countries particularly in populations with high rates of consanguineous marriages. Here, we are reporting the first data on this aspect from Sudan. This is a descriptive, retrospective, hospital-based study, carried out in the two main paediatric endocrinology centres in Sudan (Gafaar Ibn Auf Paediatric Tertiary Hospital and Soba University Hospital, Khartoum) from January 2006 up to December 2014. Patients' records were reviewed for relevant demographical, clinical, hormonal and radiological data using pretested study forms. The study included 156 patients. One hundred and one patients were males (M: F = 1.8:1). The commonest age groups were adolescents (57.7%). Consanguinity was found in 77.8% of patients overall and 91% of patients with congenital aetiologies. The commonest clinical presentation was short stature (93.5%). Congenital causes (86.5%) were more prevalent than acquired causes (13.5%). There were six family clusters with multiple pituitary hormone deficiencies (MPHD) and three families with isolated growth hormone (GH) deficiency (IGHD). Most of the congenital cases with MPHD were phenotypic for PROP1 gene mutation (77.5% of sporadic cases and 50% of familial cases). Craniopharyngioma was the commonest of the acquired causes (10.2%). GH was the most frequent hormone deficient (89.7%). Abnormal Magnetic resonance imaging brain findings were significantly seen more in MPHD in comparison to IGHD. The genetic forms of hypopituitarism in populations with high rates of consanguineous marriage like Sudan may be higher than those reported internationally. Molecular genetic studies are, therefore, highly recommended.

Autoimmune hyperthyroidism in children & adolescents in Sudan: a 13 years' experience of a Paediatric Endocrinology Clinic.

Background Little has been published about hyperthyroidism in children from Sudan or Africa. In limited resource countries, lack of facilities and sociocultural factors might make international diagnosis and management guidelines difficult to follow. We aimed to determine the magnitude of autoimmune hyperthyroidism, clinical presentation, diagnosis, management and its outcome in Sudan. Method Records of all patients diagnosed as Graves' disease (GD) or Hashitoxicosis (HTx) were reviewed and missing data filled by interviewing patients and/or their families. Data including age, sex, clinical presentation, investigations, management and outcome were obtained. Results Eighty-eight patients, 80 with GD (F:M = 4.7:1) and 8 with HTx (F:M = 7:1), were diagnosed at 11.8 ± 3.05 and 11.23 ± 2.78 years, respectively (p = 0.52). GD patients were diagnosed based on presence of exophthalmos (66.25%), positive thyroid receptor autoantibodies (12.5%), prolonged illness duration (8.75%) or remission failure to only B blocker (16.25%). All GD patients were started on carbimazole and cumulative remission rate was 11.8%, 32.4% and 41.2% by end of the second, third and fourth year respectively, however it plateaus after that. While 12 GD patients underwent surgery, only three opted for radioiodine ablation. Conclusion Hyperthyroidism is not an uncommon problem. In absence of laboratory facilities, differentiation between GD and HTx can be made based on clinical grounds. Continuation of medical treatment for 4 years can increase the remission rate to 41.2%. In Sudan, surgery is the preferred method of definitive therapy.

The Impact and Successes of a Paediatric Endocrinology Fellowship Program in Africa.

Background. The prevalence and distribution of endocrine disorders in children in Africa are not well known because most cases are often undiagnosed or diagnosed too late. The awareness of this led to the launch of the Paediatric Endocrinology Training Center for Africa (PETCA) designed to improve quality and access to health care by training paediatricians from Africa in paediatric endocrinology. Methods. The fellowship is undertaken over an 18-month period: six months of clinical and theoretical training in Kenya, nine months of project research at the fellow's home country, and three months of consolidation in Kenya. Upon completion, certified paediatricians are expected to set up centers of excellence. Results. There have been two phases, phase I from January 2008 to October 2012 and phase II from January 2012 to April 2015. Fifty-four fellows from 12 African countries have been certified, 34 (phase I) and 20 (phase II). Over 1,000 patients with wide ranging diabetes and endocrine disorders have been diagnosed and treated and are being followed up at the centers of excellence. Conclusion. The successes of the PETCA initiative demonstrate the impact a capacity building and knowledge transfer model can have on people in resource-poor settings using limited resources.